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Isolated left ventricular noncompaction in a newborn with Pierre-Robin sequence

Pediatric Cardiology · Şubat 2013

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YÖKSİS Kayıtları
Isolated Left Ventricular Noncompaction in a Newborn With Pierre Robin Sequence
Pediatric Cardiology · 2013 SCI-Expanded
Prof. Dr. AHMET SERT →
YÖKSİS ISSN Eşleşmesi

Bu dergide (ISSN eşleşmesi) kurumun 11 kaydı bulundu.

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Unusually Prominent Chiari s Network Prolapsing Into the Right Ventricle in an Asymptomatic Newborn
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Isolated Left Ventricular Noncompaction in a Newborn With Pierre Robin Sequence
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Temporary Cardiac Pacemaker in the Treatment of Junctional Rhythm and Hypotension Due to Imipramine Intoxication
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Assessment of Structure, Function, and Rhythm of the Heart with Echocardiography and Electrocardiography in Adolescent Swimmers
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Assessment of Structure, Function, and Rhythm of the Heart with Echocardiography and Electrocardiography in Adolescent Swimmers
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One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study
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One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study
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Makale Bilgileri

Dergi Pediatric Cardiology
ISSN01720643
Yayın TarihiŞubat 2013
Cilt / Sayfa34 · 452-454
DOI10.1007/s00246-012-0294-8
Scopus ID2-s2.0-84879497897
Özet Pierre-Robin sequence or syndrome (PRS) (OMIM #261800) is characterized by a small mandible (micrognathia), posterior displacement/retraction of the tongue (glossoptosis), and upper airway obstruction. It has an incidence varying from 1 in 8,500 to 1 in 30,000 births. Congenital heart defects (CHDs) occur in 20 % of the patients with PRS. Ventricular septal defect, patent ductus arteriosus, and atrial septal defects are the most common lesions. Noncompaction of the ventricular myocardium is a rare cardiomyopathy characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses. It is thought to be caused by arrest of the normal endomyocardial morphogenesis. Isolated left ventricular noncompaction (LVNC) in patients with PRS has not been reported previously. This report describes a newborn with PRS and isolated LVNC. Previously, LVNC has been reported in association with mitochondrial disorders, Barth syndrome hypertrophic cardiomyopathy, zaspopathy, muscular dystrophy type 1, 1p36 deletion, Turner syndrome, Ohtahara syndrome, distal 5q deletion, mosaic trisomy 22, trisomy 13, DiGeorge syndrome, and 1q43 deletion with decreasing frequency. Karyotype analysis of the reported patient showed normal chromosomes (46, XX), and a fluorescent in situ hybridization study did not show chromosome 22q11.2 deletion. This is the first clinical report of a patient with isolated LVNC and PRS. Noncompaction of the ventricular myocardium is a rare and unique disorder with characteristic morphologic features that can be identified by echocardiography. Long-term follow-up evaluation for development of progressive LV dysfunction and cardiac arrhythmias is indicated for these patients. © 2012 Springer Science+Business Media, LLC.

Yazarlar (5)

1
Ebru Aypar
2
Ahmet Sert
ORCID: 0000-0002-1607-7569
3
Zeynel Gökmen
4
Eyup Aslan
5
Dursun Odabas

Anahtar Kelimeler

Left ventricular noncompaction Noncompaction Noncompaction of ventricular myocardium Pierre-Robin sequence

Kurumlar

Konya Meram Training and Research Hospital
Konya Turkey
Scimago Dergi (ISSN Eşleşmesi)
Pediatric Cardiology
Q2
SJR Skoru0,586
H-Index90
YayıncıSpringer
ÜlkeUnited States
Cardiology and Cardiovascular Medicine (Q2)
Pediatrics, Perinatology and Child Health (Q2)
Dergi sayfasına git

Metrikler

5
Atıf
5
Yazar
4
Anahtar Kelime

Sistemimizdeki Yazarlar

AS
AHMET SERT
Prof. Dr.

Hızlı Erişim

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